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Frequency of Death Without Parenteral Prostaglandin Therapy in Pulmonary Arterial Hypertension Patients at a Tertiary Referral Center

Bryan Hay

Meredith Pugh

Ivan Robbins

Anna Hemnes


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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Bryan Hay

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Background: Parenteral prostaglandin (PP) therapy has been shown to increase exercise tolerance, improve quality of and prolong life in patents with pulmonary arterial hypertension (PAH). Despite this, many patients with PAH die without PP therapy according to recent data from the REVEAL registry. We sought to examine causes of death in PAH patients not on PP therapy with they hypothesis that most of these patients died of non-PAH causes.

Methods: A single center retrospective cohort analysis of consecutive patients evaluated for pulmonary hypertension at our Center from 1/1/08 to 12/31/12. We considered PP use to be IV epoprostenol/treprostinil or SQ treprostinil. Disease severity, duration, hemodynamics, and cause of death were compared between PAH patients treated with PP (PAH-PP) and those who were not, but were not documented as poor PP candidates (PAH-nonPP).

Results: Of 1786 patients evaluated at our center, 102 were diagnosed with PAH and died. Of these, 61 were PAH-PP, 41 were not treated with PP, 28 were documented by physicians in the medical record to be poor candidates for PP and 13 patients were not documented to be poor candidates, but did not receive PP therapy (PAH-nonPP). Age, sex, medical co-morbidities, PAH sub-type and NYHA functional class were not different in the PAH-PP group versus PAH-nonPP, however time from diagnosis to worst functional class was longer in the PAH-nonPP than the PAH-PP group (median days 301 vs. 833, p<0.05).  PAH-nonPP patients had lower right atrial pressure (7.6 ± 2.8 vs. 12.8 ± 6.0 mmHg, p<0.05), but were not different by other hemodynamic measures. PAH-PP patients more frequently experienced their worst functional class within one year of diagnosis (34/61 vs. 2/13, p<0.05) and were less commonly treated with combination oral therapy (p<0.05). Of the 13 PAH-nonPP patients, four refused prostaglandin treatment and three had other life-limiting conditions (dialysis (2), metastatic cancer (1)). Of the remaining patients, cause of death was sudden cardiac death (3), gastrointestinal hemorrhage (2), and pneumonia (1).  

Conclusions: At a large referral center, 13% of PAH patients died without PP therapy but would otherwise be considered candidates for PP. PAH-nonPP had longer duration of disease and more combination therapy with less right heart failure, suggesting greater challenges in identification of patients at risk of death. Our findings are different from those reported in the REVEAL registry and indicate that the majority of patients with advanced PAH who are appropriate candidates receive PP therapy.

Type: Clinical Science